The relation between hyperosmolality induced by the alkali therapy and activities of central nervous system was studied in 45 rabbits (weighing 1.0-2.0kg). An electrophysiological observation was made on the threshold of arousal reaction and evoked muscular discharges following the stimuli (100 Hz) on the midbrain reticular formation. Spontaneous electroencephalography and electromyography were done as well. Hypertonic solutions (7% sodium bicarbonate, 4.64% sodium chloride, 20% glucose and 20% mannitol) were kept infused into the ear vein with dripping rate between 20 and 30 ml/kg/hr.
When the plasma osmolality exceeded 370 mOsm/L with the infusion of 7% sodium bicarbonate, intracranial hemorrhage (ICH) began to take place. The mean lethal osmolality read 441. When 4.64% sodium chloride and 20% glucose were used, ICH was seen at as high an osmolality as 491.7 and 479.0 mOsm/L in plasma respectively, which were higher than when 7% sodium bicarbonate was used (p<0.05).
The electroencephalogram showed spikes and spike bursts in accordance with convulsions, jerks and other neurological signs at the osmolality over 400. Such phenomena were not manifest, however, when 7% sodium bicarbonate solution was given.
The patterns shown in electroencephalogram and electromyogram were categorized into the following 6 stages when 7% sodium bicarbonate was used. The stage 0 was for normal pattern, the stage 3 for moderate brain damage as the arousal wave was not observed when the plasma osmolality was 330-350 mOsm/L. The stage 5 was shown flat pattern, exceeded 370 mOsm/L in plasma.
The threshold of arousal reaction was elevated by 7% sodium bicarbonate and 4.64% sodium chloride. Elevation of the threshold of evoked muscular discharge was induced only by 7% sodium bicarbonate.
From these results, it was concluded that hyperosmolality was obviously one of the causes of ICH, but other factors must also be related to it. The convulsions or other neurological signs caused by hyperosmolality probably arose from discharges at the cortex or subcortex. The disturbance in consciousness by acute hyperosmolality was influenced by sodium ions. The depression of motor system was not seen except when 7% sodium bicarbonate wasinfused.

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The authors discussed a characteristic pattern of cerebral circulation seen by carotid angiography in the newborn babies within a month after birth.
The arterial phase was much longer than in older infants. Consequently, the venous phase started later, with a specific order of appearance. The deep veins appeared 1.0-3.0 seconds earlier than the superficial veins, 2.0-5.5 seconds and 3.5-8.5 seconds respectively after the onset of the arterial phase. This specific order of appearance of the veins seemed to be connected with good visualization of the basal gangllia and the choroid plexus.
It was concluded that a long circulation time and the order of appearance of the veins were characteristic of the angiographic findings at the neonatal period.

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Prostaglandin was shown to be pyrogenic in central nervous system of animals and vasoconstrictor effect of prostaglandin F₂α on the carotid artery has been reported. These studies suggested that prostaglandin F₂α of the central nervous system may be related to the pathogenesis of febrile convulsion. The levels of cerebrospinal fluid prostaglandin F₂α were monitored using radioimmunoassay in children as follows: febrile convulsion (18 cases); meningitis (23 cases); epilepsy (18 cases) and non-neurological diseases (14 cases). We examined the cerebrospinal fluid in children with non-neurological diseases because of suspected neurological diseases at admission time.
A seven-fold increase in the level of prostaglandin F₂α was found in patients with febrile convulsion when their body temperature was above 37.5°C to 40°C, as compared to normal leves of prostaglandin F₂α in children with non-neurological diseases and body temperature above 37.5°C to 40°C (293.9 pg/m/ as compared to an average of 42.0 pg/m/). The prostaglandin F₂α levels were high within a day after the episodes of febrile convulsions (average value 329.3 pg/m/) and dramatically decreased after the first day (average value 41.8 pg/ml). A three-fold increase of prostaglandin F₂α levels was also found in children with meningitis but an increase of prostaglandin F₂α levels was not found in children with epilepsy when their body temperature was above 37.5°Cto 40°C.
In view of these results the most suitable therapy would be the administration of aspirin or indomethacin to antagonize the effect of prostaglandin, in conjuction with the usual antiepileptic drugs.

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A neuroanatomical study of cholinergic and aminergic systems innervating the amygdala, an important limbic structure for the pathogenesis of epilepsy, has been made by using the catecholamine fluorescence histochemistly or histochemical method for cholinergic systems in conjunction with the retrograde axonal transport techniques.
Dopaminergic amygdalopedal fibers were derived from mesencephalic dopamine cell groups; the dorsal part of of the pars compacta of the substantia nigra and the peripeduncular nucleus as well as dorsal part of the ventral tegmental area (Tsai). Cholinergic fibers in the amygdala were originated from a magnocellular group situated in the substantia innominata, which was identified to be a part of the basal nucleus (Meynert). Although cholinergic neurons in the substantia innominata also sent their axons to the cerebral cortex, it appeared that such neurons had no divergent projections to the amygdala.

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In 29 infants with infantile spasms, development quotients of auditory behaviors (DQ), thresholds of conditioned orientation reflex audiometry (COR), auditory brain stem responses (ABRs), slow vertex responses (SVR) and visual evoked responses (VER) were studied to determine the level of the lesion causing their hearing disorders. Seventy six percent of infants with infantile spasms showed severely delayed development quotients (below 33%), and 86.2% of these were found to have a moderate elevation of the COR threshold. ABR thresholds, peak latencies of waves I and V, and the interwave latency between waves I and V were measured. 27.6% of our cases were found to have the ABR abnormalities. One case (3.4%) had peripheral hearing loss and 24.2% had brain stem lesions. Cerebral evoked responses were evaluated as positive when reproducible responses were recorded. Slow vertex responses to 85-dB click stimuli were present in 20.7% and photically visual evoked responses were present in 41.4% of cases. These results revealed that a fourth Of our cases had brain stem lesions at least, but that others, except an infant with peripheral hearing loss, had essentially normal function at the brain stem level even though they responded to sound stimuli as poorly as deaf infants. In conclusion, a single lesion site in the auditory nervous systems of infnats with infantile spasms was not identified. Each case might have some variable lesions between the lower brain stem and cerebrum.

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Human behavior is considered to be mediated by neurotransmitters in the brain. The measurement of the precursors and metabolites of neurotransmitters in cerebrospinal fluid gives an indication of the turnover of these substances and the most direct method available for assessing chemical transmission in the central nervous system. Tryptophan (Trp) and tyrosine (Tyr) are precursors for the brain neurotransmitters serotonin and dopamine, respectively. The major brain metabolite of serotonin is 5-hydroxyindoleacetic acid (5-HIAA), whereas dopamine is predominantly metabolized to homovanillic acid (HVA). The present study was carried out to examine developmental changes of 5-HIAA, HVA, Trp and Tyr in the lumbar CSF. The experiments were performed on 50 samples without central nervous system diseases or administration of anticonvulsants or hypnotics.
5-HIAA and HVA in CSF were determined by the liquid chromatographic-amperometric detectors. The mean values±SD (ng/ml) of 5-HIAA concentrations on lumbar CSF were 138.3±33.5 in full-term neonates (n=8), 39.3±5.5 in infants (n=9), 24.7±9.7 in young children (n=14) and 13.8±9.3 in school and pubescent children (n=13). The difference between full-term neonates and infants was highly significant (PG 0.01 in t-test). The mean values±SD (ng/ml) of HVA concentrations in lumbar CSF were 167.0±64.6 in full-term neonates (n=8), 112.3±48.4 in infants (n=9), 106.5-28.8 in young children (n=14) and 46.2±28.1 in school and pubescent children (n=13). The difference between full-term neonates and school and pubescent children was significant (P<0.05 in t-test). The values of Trp and Tyr also decreased with increasing age. The correlation coefficient between Trp and 5-HIAA was 0.644 (P<0.01) and that between Tyr and HVA was 0.43 (P<0.01). The ratios of Trp to 5-HIAA and Tyr to HVA did not reveal developmental changes.
All findings support the theory that the cause of the increased level of 5-HIAA and HVA is highly dependent on the increased uptake of the precursors into the brain.

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A case of ring chromosome 6 was presented as the first case in Japan. Clinical and cytogenetic studies were carried out on a l-year-six-month-old boy with epileptic seizures, psychomotor retardation, polydactylia, small head, epicanthus and hypertelorism.
The parents were non consanguineous. The delivery was normal and his birth weight was 2, 370g. His initial motor development was almost normal until about 10 months after birth. At 8 months after birth he developed nuchal atonic seizures (neck flexon toward right foreward) with eye ball elevation. These attacks were seen five to six times per hour only on waking and did not occur in series.
Physical status on admission revealed a small boy with slightly apathetic facial appearance. DQ was 50 (Tsumori-Inage method). Routine laboratory examination revealed no abnormalities. The C-banding technique showed a ring 6 chromosome, with the break points located on bands p25 and q27. By C-band staining 80 per cent of cells showed the monocentric ring and 20 per cent of cells showed the dicentric ring.
Only nine cases of ring chromosome 6 (including propositus) have been reported in the world. Specific clinical manifestations are not yet known in this disease. Polydactylia and intractable seizures with hypsarhythmia have not been recorded in the previous cases. The seizures were not typical of infantile spasms, and could not be controlled completely although the frequency of attacks was reduced by PB, DPA, BZP and Vitamin B6.

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A male infant developed hypotonia at 5 months, vomiting, diarrhea, fever, generalized clonic convulsion, tonic spasm and periodical opisthotonus at 8 months, swallowing difficulty at 10 months, pes equinovarus and optic atrophy at 11 months, and then tachypnea, and died at 14 months of age. Parents were consanguinous. Laboratory studies revealed elevated serum LDH, CPK, lactate and Pyruvate. TPP-ATP phosphoryl transferase inhibitor was negative in urine. EEG showed irregular and diffuse slow waves and periodic diffuse spike and waves. CT scan at 9 months of age showed slightly low attenuation areas in the putamen bilaterally.
At 11 months, a diffuse cerebral atrophy was found, and the low attenuation of the basal ganglia became more definite. No enhanced lesion was seen at 13 months of age. Thiamine tera -hydrofurfuryl disulfide and lipoic acid were tried without success.
The pathological findings of the brain were astrogliosis and proliferation of capillaries in putamen, thalamus, caudate neucleus, substantia nigra, pontine brachium and cerebral cortex, which were symmetrically involved. The symmetrical cavitation was found in putamen. Optic nerve and mamillary body were spared. CT scan findings corresponded well with the pathology of the necrotic lesions of the brain. It was concluded that these CT scan pictures described above may be diagnostic of Leigh syndrome.

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